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BACKGROUND: There is little evidence on managing the proximal aorta of 4.0-4.5 cm during aortic valve replacement (AVR) in bicuspid aortic valve (BAV) patients. METHODS: A total of 431 patients between 1993-2019 underwent either an isolated AVR, AVR + concomitant ascending aorta replacement, or aortic root replacement. We divided patients into native root dilation [4.0-4.5 cm, n=121] vs. root control groups [

BACKGROUND: Main-duct (MD) intraductal papillary mucinous neoplasm (IPMN) is associated with malignancy risk. There is a lack of consensus on treatment (partial or total pancreatectomy) when the MD is diffusely involved. We sought to characterize the pancreatic remnant fate after partial pancreatectomy for non-invasive diffuse MD-IPMN. METHODS: Consecutive patients with partial pancreatectomy for non-invasive MD-IPMN from 2004 to 2016 were analyzed. Diffuse MD-IPMN was defined by preoperative imaging as dilation of the MD in the head of the pancreas more than 5mm and involving the whole gland. RESULTS: Of 127 patients with resected non-invasive MD-IPMN, 47 (37%) had diffuse MD involvement. Eleven of 47(23%) patients developed imaging evidence of progression or new cystic disease in the pancreatic remnant. Patients with diffuse MD-IPMN were older (73yrs vs 67yrs, p=0.009), more likely to receive a pancreaticoduodenectomy (96% vs 56%, p

Post-Refractive Surgery Ectasia is a serious, sight-threatening, and highly - avoided complication seen after the following procedures: Laser in situ Keratomileusis, Photorefractive Keratectomy, Small Incision Lenticule Extraction, Radial and/or Arcuate Keratotomy. Specific risk factors may include age, corneal thickness, degree of refractive error, corneal topographic changes including irregular astigmatism, percent tissue ablation, and residual stromal bed. Biomarkers may be a new option to help indicate who is at greatest risk for ectasia. Visual aids including glasses or contacts lenses are often required to achieve optimal vision. Collagen crosslinking is the only treatment thought to stop progression of ectasia and prevent keratoplasty. Other surgical options may include topography-guided phototherapeutic keratectomy and intrastromal corneal ring segments. Ultimately an "ounce of prevention is a pound of cure" so careful preoperative screening and ultimately offering the safest and most effective treatments for patients is arguably the most important job of the refractive surgeon.

Objective Aortic aneurysm and dissection are major life-threatening complications of Marfan syndrome. Avoiding factors that promote aortic damage is critical in managing the care of these patients. Findings from clinical and animal studies raise concerns regarding fluoroquinolone use in patients at risk for aortic aneurysm and dissection. Therefore, we examined the effects of ciprofloxacin on aortic aneurysm and dissection development in Marfan mice. Methods Eight-week-old Marfan mice (Fbn1C1041G/+) were given ciprofloxacin (100 mg/kg/d; n = 51) or vehicle (n = 59) for 4 weeks. Mice were monitored for 16 weeks. Aortic diameters were measured by using ultrasonography, and aortic structure was examined by using histopathologic and immunostaining analyses. Results Vehicle-treated Fbn1C1041G/+ mice showed progressive aortic enlargement, with aortic rupture occurring in 5% of these mice. Compared with vehicle-treated Fbn1C1041G/+ mice, ciprofloxacin-treated Fbn1C1041G/+ mice showed accelerated aortic enlargement (P = .01) and increased incidences of aortic dissection (25% vs 47%, P = .03) and rupture (5% vs 25%, P = .005). Furthermore, ciprofloxacin-treated Fbn1C1041G/+ mice had higher levels of elastic fiber fragmentation, matrix metalloproteinase expression, and apoptosis than did vehicle-treated Fbn1C1041G/+ mice. Conclusions Ciprofloxacin accelerates aortic root enlargement and increases the incidence of aortic dissection and rupture in Marfan mice, partially by suppressing lysyl oxidase expression and further compromising the inherited defect in aortic elastic fibers. Our findings substantiate that ciprofloxacin should be avoided in patients with Marfan syndrome.

We describe a case of congenital giant megaureter (CGM) in a sixteen-year-old female. She presented with a five-day history of abdominal distention, right flank pain and tenderness. Right pyelonephritis was suspected. Computerized tomography (CT) showed a large cystic abdominal mass with no appreciably functioning left kidney causing secondary compression of the contralateral right ureter. A left upper nephroureterectomy was performed, draining over 3.5 L of fluid. Our experience suggests that CGM should be considered in the differential for pediatric patients presenting with a cystic abdominal mass.

Tricuspid regurgitation is common in patients with severe degenerative mitral regurgitation. However, the evidence base is insufficient to inform a decision about whether to perform tricuspid-valve repair during mitral-valve surgery in patients who have moderate tricuspid regurgitation or less-than-moderate regurgitation with annular dilatation.We randomly assigned 401 patients who were undergoing mitral-valve surgery for degenerative mitral regurgitation to receive a procedure with or without tricuspid annuloplasty (TA). The primary 2-year end point was a composite of reoperation for tricuspid regurgitation, progression of tricuspid regurgitation by two grades from baseline or the presence of severe tricuspid regurgitation, or death.Patients who underwent mitral-valve surgery plus TA had fewer primary-end-point events than those who underwent mitral-valve surgery alone (3.9% vs. 10.2%) (relative risk, 0.37; 95% confidence interval [CI], 0.16 to 0.86; P = 0.02). Two-year mortality was 3.2% in the surgery-plus-TA group and 4.5% in the surgery-alone group (relative risk, 0.69; 95% CI, 0.25 to 1.88). The 2-year prevalence of progression of tricuspid regurgitation was lower in the surgery-plus-TA group than in the surgery-alone group (0.6% vs. 6.1%; relative risk, 0.09; 95% CI, 0.01 to 0.69). The frequencies of major adverse cardiac and cerebrovascular events, functional status, and quality of life were similar in the two groups at 2 years, although the incidence of permanent pacemaker implantation was higher in the surgery-plus-TA group than in the surgery-alone group (14.1% vs. 2.5%; rate ratio, 5.75; 95% CI, 2.27 to 14.60).Among patients undergoing mitral-valve surgery, those who also received TA had a lower incidence of a primary-end-point event than those who underwent mitral-valve surgery alone at 2 years, a reduction that was driven by less frequent progression to severe tricuspid regurgitation. Tricuspid repair resulted in more frequent permanent pacemaker implantation. Whether reduced progression of tricuspid regurgitation results in long-term clinical benefit can be determined only with longer follow-up. (Funded by the National Heart, Lung, and Blood Institute and the German Center for Cardiovascular Research; ClinicalTrials.gov number, NCT02675244.).

Background: Former studies showed that magnetic resonance cholangiopancreatography (MRCP) is useful in diagnosing the presence of santorinicele; however, few studies have evaluated MRCP in diagnosing wirsungocele and the association between pancreatitis and santorinicele or wirsungocele. The purpose of the study was to explore the performance of MRCP in diagnosing santorinicele and wirsungocele and investigate the potential association among pancreatitis, pancreas divisum, and santorinicele or wirsungocele. Method: Sixty-five patients (mean age, 55.68 years; range, 11-82 years) with santorinicele or wirsungocele were included and sorted into two groups: the santorinicele group (n = 48) and the wirsungocele group (n = 17). All patients underwent MRCP. The images were evaluated for the appearance and size of santorinicele or wirsungocele. The diagnostic sensitivity of MRCP was assessed. Additionally, whether two groups are correlated with pancreas divisum or pancreatitis were investigated. Result: The sensitivity of MRCP in detecting santorinicele and wirsungocele showed no difference (68.8% and 76.5%, respectively). The proportion of patients who developed pancreatitis in santorinicele and wirsungocele groups were 60.4% and 11.8%, respectively (p < 0.05). Pancreas divisum accounted for 77.1% and 11.8% of the patients in the santorinicele and wirsungocele groups, respectively (p < 0.05). Patients with santorinicele and pancreas divisum tended to be older when they acquired pancreatitis. Conclusion: MRCP could be an alternative imaging method to detect cystic dilation of the pancreatic duct. Pancreatitis is more common in patients with santorinicele than in those with wirsungocele. Moreover, santorinicele is more closely associated with pancreatitis than with pancreas divisum.

Aneurysms of the ascending aorta, unrelated to xenobiotic administration, are described in 5 rats and 2 mice in nonclinical safety studies conducted at Charles River Laboratories (CRL) sites over the past 10 years. The most prominent microscopic finding was focal dilation with disruption of the wall of the ascending aorta with chronic adventitial inflammation or fibroplasia. The pathogenesis of this finding is unknown. There were no associated macroscopic findings, clinical abnormalities, or vascular lesions elsewhere. The results of a search of historical control data from toxicology studies of 1 day to 72 weeks’ duration performed at CRL for aortic findings from 5900 mice and 23,662 rats are also reported. Aortic lesions are uncommon in mice and rats used in nonclinical safety studies, but toxicologic pathologists should be aware that aneurysms of the ascending aorta with fibroplasia and inflammation in the aortic wall and adventitia may occur spontaneously or iatrogenically, as they have the potential to impact interpretation in toxicology studies.

Personalized External Aortic Root Support (PEARS) is an evolving method of treatment for patients with a dilated aortic root or ascending aorta. This treatment is being adopted in an increasing number of centres. For the sake of the safety of the procedure a standardized surgical technique is necessary. The authors describe a surgical technique of implantation that is derived from their extensive experience.

BACKGROUND. Drug-eluting bead transarterial chemoembolization (DEB-TACE) has emerged as an alternative to conventional TACE (cTACE) for treatment of hepatocellular carcinoma (HCC), although selecti...

Objective Thoracic endovascular aortic repair (TEVAR) has become a mainstay of treatment for a variety of thoracic aortic pathologies. Expansion of the proximal aortic neck after endovascular repair of abdominal aortic aneurysms has been demonstrated; however, dilatation of the proximal aortic neck after TEVAR has not been well described. We sought to describe remodeling of the proximal neck following TEVAR. Methods This is a retrospective, single institution review of patients who underwent TEVAR for thoracic aortic aneurysm (TAA) and dissection with aneurysmal degeneration from 2010 to 2019. Postoperative computed tomography scans were reviewed and aortic diameter was measured in orthogonal planes using 3-dimensional centerline reconstruction software. The primary outcome was change in aortic diameter at the proximal aortic neck as compared to the initial postoperative computed tomography scan. Clinical and operative data were analyzed to identify factors associated with significant neck dilatation. Results Of 87 patients who underwent TEVAR during the study period, 30 met inclusion criteria. Median follow up was 20.5 months. Median age was 67 years, and 15 patients (50%) were female. The proximal aortic neck experienced an overall increase over time in aortic diameter. Five mm distal to the graft showed the greatest rate of expansion, with a median increase of 1.3, 2.9, and 6.2 mm at one year, two years, and three years, respectively. When comparing patients who had mean expansion at this location of >2.0 mm/year to patients who did not, a higher percentage had dissection pathology (81.8% vs. 31.6%, P = 0.008), had graft placement at aortic landing zone 2 (36.4% vs. 5.3%, P = 0.028), and were smokers (100% vs. 52.6%, P = 0.006). Higher percent oversizing was shown to be associated with significant aortic neck dilatation for true aneurysms only. Conclusions Aortic neck dilatation occurs over time for the majority of patients following TEVAR with the distal neck experiencing the highest rate of expansion. Dissection pathology, aortic landing zone 2, and smoking were found to be associated with a higher rate of neck dilatation.

Ectatic corneal diseases are a group of eye disorders characterized by progressive thinning and outward bulging of the cornea, resulting in vision impairment. A few attempts have been made to use cornea-derived extracellular matrix hydrogels for corneal tissue engineering; however, no studies have investigated its application in corneal ectasia. In this study, we have first developed an animal surgical model that mimics a few specific phenotypes of ectatic cornea. Later, we investigated the potential of decellularized cornea matrix hydrogels (dCMH) from both human and bovine sources in increasing the thickness of the cornea in the developed surgical model. Our data advocate that surgical stromal depletion can be followed to establish ectatic models and can also provide information on the biocompatibility of materials, its integration with native stroma, degradation over time, and tissue remodeling. We observed that dCMH from both sources could integrate with ectatic thin corneal stroma and helps in regaining the thickness by regenerating a reasonably functional and transparent stroma; however, no significant difference was spotted between the dCMH made from human and bovine corneal tissue sources. Hence, this study is a promising step toward developing a non-invasive technique for the treatment of corneal ectasia by using dCMH.

Background Kawasaki disease (KD), which was first described by Tomisaku Kawasaki in 1967, is a syndrome that results in acute systemic vasculitis, affecting mainly infants and children, and is a major cause of acquired heart disease in developed countries. KD is diagnosed based on certain characteristic symptoms and echocardiogram results. It has been reported that neck and abdominal ultrasound is also reliable diagnostic method. Nevertheless, abdominal ultrasound is not a routine procedure in KD. Moreover, dilatation of the common bile duct (CBD) has been rarely reported in previous cases. Case presentation: A 4-year-old boy presented with fever and markedly high transaminase level (AST, 5,323 U/L, ALT, 1,554 U/L). The patient was diagnosed as having KD based on characteristic symptoms and echocardiogram findings. Neck and abdominal ultrasound revealed dilatation of the CBD as well as cervical lymphadenopathy resembling a cluster of grapes, thickening of the gallbladder wall, and increased periportal echogenicity throughout the liver parenchyma. The patient received initial treatment with intravenous immunoglobulin (IVIG) at day 4 of fever, following which, transaminase level decreased. The patient received second-line treatment with IVIG and prednisolone because of recurrent fever on day 6. Dilatation of the CBD was improved from 6.6 mm on day 4 to 3.1 mm on day 8. Although re-dilatation was observed, it gradually diminished and normalized (4.3 mm on day 28, 4.0 mm on day 63, 3.3 mm on day 105, and 2.8 mm on day 182). Conclusion This case highlights the usefulness of abdominal ultrasound and the importance of considering dilatation of the CBD as one of the complications of KD. Because abdominal ultrasound is not a routine procedure in KD, it is possible that dilatation of the CBD in KD is quite latent if not investigated thoroughly. Further prospective studies to support these findings are warranted.

Purpose Pachychoroid spectrum disease encompasses a set of macular disorders secondary to an abnormally thick choroid. However, the pathological process underlying pachychoroid spectrum disease and its overlap with age-related macular degeneration (AMD) remain unclear. This review aimed to understand the underlying pathology, classification, and phenotypes of pachychoroid spectrum disease. Methods This comprehensive literature review was performed based on a search of peer-reviewed published papers relevant to the current knowledge of pachychoroid disease spectrum. Results Pachychoroid is primarily a bilateral phenomenon; the main pathological lesions include choriocapillaris attenuation and abnormally dilated pachyvessels. Chronic central serous chorioretinopathy (CSC) and pachychoroid neovasculopathy (PNV) show similar morphological changes and angiogenic cytokine levels. The subretinal fluid in PNV may not accurately indicate PNV activity. Besides, types 1 and 2 of choroidal neovascularization (CNV) may be involved in primary pachychoroidal disease. Both choroidal arteriosclerosis and higher hydrostatic pressure contribute to hyalinized choroidal arteries and aneurysmal dilatations, resulting in PNV progression to polypoidal choroidal vasculopathy (PCV). Thus, pachychoroid-related type 2 CNV and chronic CSC could be considered as PNV (IIIc) and as a precursor of PNV (IIIa), respectively. Tangled PCV on optical coherence tomography angiography that fails to develop aneurysms should be classified as a subtype of PNV or a forme fruste of PCV. Conclusions Multiple disorders of the pachychoroid spectrum are considered as a continuous disease process, ultimately stimulated by choroidal malfunction. PCV overlaps both AMD and pachychoroid disease, especially for thin-choroid and bilateral types. The terminology and classification of pachychoroid spectrum disease should be used cautiously.

Aortic aneurysm (AA) remains one of the primary causes of death worldwide. Of the major treatments, prophylactic operative repair is used for AA to avoid potential aortic dissection or rupture. To halt the development of AA and alleviate its progression into aortic dissection, pharmacological treatment has been investigated for years. Currently, β-adrenergic blocking agents, losartan, irbesartan, angiotensin-converting-enzyme inhibitors, statins, antiplatelet agents, doxycycline, and metformin have been investigated as potential candidates for preventing AA progression. However, the paradox between preclinical successes and clinical failures still exists, with no medical therapy currently available for ideally negating the disease progression. This review describes the current drugs used for pharmacological management of AA and their individual potential mechanisms. Preclinical models for drug screening and evaluation are also discussed to gain a better understanding of the underlying pathophysiology and ultimately find new therapeutic targets for AA.

We evaluated choroidal congestion using multimodal imaging in pachychoroid neovasculopathy (PNV). In a retrospective case series of 100 eyes of 99 treatment-naïve PNV patients, their clinical records were reviewed and the corresponding multimodal imaging studies were analyzed. We assessed areas of choriocapillaris filling delay which overlapped with dilated outer choroidal vessels, choroidal neovascularization (CNV), and retinal pigment epithelium (RPE) atrophy. The study subjects were 78 men (78.8%) and 21 women (21.2%). The mean patient age was 67.5 ± 10.5 years. On indocyanine green angiography, all eyes showed choriocapillaris filling delay in the early phase. Dilated outer choroidal vessels were demonstrated in all eyes by en face optical coherence tomography. The areas of choriocapillaris filling delay overlapped extensively with that of dilated outer choroidal vessels. All eyes showed CNV localized within the sites of choriocapillaris filling delay. RPE atrophy was noted in 71 eyes (71.0%), and 68 of these (95.8%) had RPE atrophy within the areas showing choriocapillaris filling delay. These findings indicate that chronic choriocapillaris ischemia secondary to vortex vein congestion may lead to CNV development as well as RPE atrophy in eyes with PNV.

Background:Coronavirus disease 2019 (COVID-19) is responsible for significant lung disease in adults. Despite mild manifestations in most children, multisystem inflammatory syndrome (MIS-C) associated with COVID-19 is well described in older children with cardiac manifestations. However, MIS-C-related cardiac manifestations are not as well described in younger children.Methods:The study is a retrospective analysis of MIS-C patients under the age of 5 years admitted between May and November 2020 to a single centre. Included cases fulfilled the case definition of MIS-C according to Royal College of Pediatrics and Child Health criteria with laboratory, electrocardiogram, or echocardiographic evidence of cardiac disease. Collected data included patients’ demographics, laboratory results, echocardiographic findings, management, and outcomes.Results:Out of 16 MIS-C cases under 5 years of age, 10 (62.5%) had cardiac manifestations with a median age of 12 months, 9 (90%) were previously healthy. Cardiac manifestations included coronary arterial aneurysms or ectasia in five (50%) cases, two (20%) with isolated myopericarditis, coronary ectasia with myocarditis in two (20%), and supraventricular tachycardia in one (10%). Intravenous immunoglobulins were given in all cases with coronary involvement or myocarditis. The median duration of hospitalisation was 7 (6–14) days; two (20%) cases with cardiac disease were mechanically ventilated and mortality in MIS-C cases below 5 years was 12.5%. Normalisation of systolic function occurred in half of the affected cases within 1 week and reached 100% by 30 days of follow-up.Conclusions:MIS-C associated with SARS-CoV-2 has a high possibility of serious associated cardiac manifestations in children under the age of 5 years with mortality and/or long-term morbidities such as coronary aneurysms even in previously healthy children.

Background Stanford type-B aortic dissection (TBAD) is commonly treated by thoracic endovascular aortic repair (TEVAR). Usually, the implanted stent-grafts will not cover the entire dissection-affected region for those patients with dissection extending beyond the thoracic aorta, thus the fate of the uncovered aortic segment is uncertain. This study used 3-dimensional measurement of aortic morphological changes to classify the different remodeling effects of TBAD patients after TEVAR, and hypothesized that not only initial morphological features, but also their change over time at follow-up are associated with the remodeling. Methods Forty-one TBAD patients underwent TEVAR and CT-angiography before and after the intervention (twice or more follow-ups) were included in this study. According to the false-lumen volume variations post-TEVAR, patients who had abdominal aortic expansion at the second follow-up were classified into the Enlarged (n =12, 29%) and remaining into the Stable group (n = 29, 71%). 3D morphological parameters were extracted on precise reconstruction of imaging datasets. Statistical differences in 3D morphological parameters over time between the 2 groups and the relationship among these parameters were analyzed. Results In the Enlarged group, the number of all tears before TEVAR was significantly higher (P = 0.022), and the size of all tears at the first and second follow-up post-TEVAR were significantly higher than that in the Stable group (P = 0.008 and P = 0.007). The location of the primary tear was significantly higher (P = 0.031) in the Stable group. The cross-sectional analysis of several slices below the primary tear before TEVAR shows different shape features of the false lumen in the Stable (cone-like) and Enlarged (hourglass-like) groups. The number of tears before TEVAR has a positive correlation with the post-TEVAR development of dissection (r = 0.683, P = 0.00). Conclusion The results in this study indicated that the TBAD patients with larger tear areas, more re-entry tears and with the primary tear proximal to the arch would face a higher risk of negative remodeling after TEVAR.

A 1-month-old nondysmorphic female infant presents with repeated episodes of abdominal distention and irregular stool patterns. Prenatal findings were significant for oligohydramnios at 34 weeks’ gestation. The infant was born at 36 weeks’ gestation with a birthweight of 2,250 g (10th percentile). She had abdominal distention and delayed passage of meconium, which occurred after a saline enema on the 3rd postnatal day and was discharged at 5 days of age. At 11 days of age, she had constipation with abdominal distention, for which she received simethicone drops that resolved her symptoms. At 26 days of age, she was admitted to a community hospital as a result of poor feeding, loose stools, and fever. She had poor perfusion and room air oxygen saturation of 88% at admission. She received intravenous fluid, antibiotics, and inotropes. Her laboratory testing showed a hemoglobin level of 13.9 g/dL (139 g/L), white blood cell count of 7,300/µL (7.3 × 109/L), platelet count of 415 × 103/µL (415 × 109/L), C-reactive protein of 0.6 mg/dL (6 mg/L), sodium of 136 mEq/L (136 mmol/L), potassium of 5 mEq/L (5 mmol/L), chloride of 103 mEq/L (103 mmol/L), and thyroid-stimulating hormone of 4.6 mIU/L. A blood culture was negative and she was presumed to have an infectious enterocolitis. Abdominal ultrasonography showed a fluid-filled distended bowel and a normal relationship of the …

Background/aim Damage to elastin fibres in coronary media might lead to coronary artery ectasia (CAE). This study evaluated whether CAE can be distinguished by detecting circulating soluble elastin (s-elastin), which is a degradation product of elastin fibres, and elastase, which is the main enzyme of elastin fibres. Materials and methods Fifty-eight patients with CAE, 58 with coronary heart disease (CHD) and 61 with relatively normal coronary arteries, were included. Circulating s-elastin and elastase were measured, and receiver operating characteristic curves were used to demonstrate their respective optimal cut-off values for predicting CAE. Results The concentrations of s-elastin and elastase were higher in the CAE group than in the CHD and relatively-normal-coronary groups. Their cut-off values for screening of CAE were 13.148 ng/mL and 25.549 ng/mL, respectively; for sensitivity of CAE were 0.690 and 0.773, respectively; and for specificity of CAE were 0.862 and 0.571, respectively. A combination of s-elastin and elastase in series (one of the two higher than its cut-off value) had a better sensitivity for screening for CAE, whereas their combination in parallel (both higher than their cut-off values) had a better specificity. Conclusion Circulating s-elastin and elastase are promising biomarkers for assisting in CAE diagnosis.

We clarified the changes in coronary artery diameters based on the degree of coronary artery involvement immediately after acute Kawasaki disease (KD). Two hundred sixteen coronary arteries in 85 patients after KD examined by two-dimensional echocardiography were reviewed from 1995. The maximal internal diameters were measured at 2 months, 1 year, 3 years, 10 years and 15 years after KD. The coronary arteries were divided into five groups based on the absolute diameter at 2 months, as well as six groups based on the Z score at 2 months. The maximum diameters were compared at 2 months with those during follow-up in each group. The numbers of right coronary, left anterior descending, left coronary, and left circumflex arteries were 84, 73, 55 and 4, respectively. There was a significant relationship between the maximum internal diameter at 2 months and subsequent changes in the maximum diameters after KD in the late period in both groups (p

OBJECTIVE To determine whether serum urate reduction with allopurinol lowers blood pressure (BP) in young adults and the mechanisms mediating this hypothesized effect. METHODS We conducted a single-center, randomized, double-blind, crossover clinical trial. Adults ages 18-40 years with baseline systolic BP ≥120 and

Background Purpose To evaluate Pentacam and OPD-Scan parameters in the early detection of keratoconus. Design Retrospective case-control study. Methods Case group included 50 clinically unaffected fellow eyes diagnosed with asymmetric keratoconus showing subtle qualitative changes at the 0.5-D sensitivity OPD-Scan scale, as well as normal anterior and back elevation difference map at Belin/Ambrosio enhanced ectasia display (BAD) at the Pentacam. Control group included 172 normal eyes that underwent Lasik surgery and presented no complications throughout the 2-year follow-up period. OPD-Scan and Pentacam parameters were compared, calculating sensitivity, specificity, and area under the receiver operating characteristic curve (AUC). A multivariate analysis was performed using Pentacam or OPD-Scan variables, and a model using variables of both devices. Results Pentacam variables with AUC ≥0.8 were keratoconus index (0.85), index of height decentration (0.81), and overall deviation at BAD (0.8). OPD-Scan variables with AUC ≥0.8 were keratoconus prediction index (0.83), surface asymmetry index (0.83), and total of higher-order trefoil aberration (0.8). In the multivariate analysis, the AUC was 0.85 in the case of OPD-Scan whereas it was 0.89 in the case of Pentacam. When combining all variables from the 2 devices, the AUC was 0.93, with a sensitivity of 82% and a specificity of 94%. Conclusions Several parameters of OPD-Scan and Pentacam can be useful to differentiate cases from normal control eyes, demonstrating even better results when combining parameters of both devices. Anterior corneal indexes were the most important parameters to discriminate both groups.

Type A aortic dissection (TAAD) involves the ascending aorta or the arch. Acute TAAD usually requires urgent replacement of the ascending aorta. However, a subset of these patients develops aortic rupture due to further dilatation of the residual dissected aorta. There is currently no reliable means to predict the risk of dilatation following TAAD repair. In this study, we performed a comprehensive morphological and hemodynamic analysis for patients with and without progressive aortic dilatation following surgical replacement of the ascending aorta. Patient-specific models of repaired TAAD were reconstructed from post-surgery computed tomography images for detailed computational fluid dynamic analysis. Geometric and hemodynamic parameters were evaluated and compared between patients with stable aortic diameters (N = 9) and those with aortic dilatation (N = 8). Our results showed that the number of re-entry tears and true/false lumen pressure difference were significantly different between the two groups. Patients with progressive aortic dilatation had higher luminal pressure difference (6.7 [4.6, 10.9] vs. 0.9 [0.5, 2.3] mmHg; P = 0.001) and fewer re-entry tears (1.5 [1, 2.8] vs. 5 [3.3, 7.5]; P = 0.02) compared to patients with stable aortic diameters, suggesting that these factors may serve as potential predictors of aneurysmal dilatation following surgical repair of TAAD.

SUMMARY OBJECTIVE: The predictive value of the fibrinogen-to-albumin ratio has been evidenced in coronary artery disease. Available data demonstrated that inflammation and oxidative stress are the relevant mechanisms of ascending aortic aneurysm formation and dilatation. The fibrinogen-to-albumin ratio reflects oxidative stress and inflammation. This study investigated the correlation between fibrinogen-to-albumin ratio and ascending aortic aneurysm. METHODS: A total of 250 consecutive patients with ascending aortic aneurysm and 250 consecutive patients with normal ascending aortic diameter were included in the study using comprehensive transthoracic echocardiography. All data and fibrinogen-to-albumin ratio were compared between two groups. RESULTS: The fibrinogen-to-albumin ratio levels were significantly higher in ascending aortic aneurysm group compared with normal ascending aortic diameter group (p

Objective: Nonatherosclerotic outward remodeling of the large intracranial arteries may facilitate propagation of excessive aortic pulsatile energy into the cerebral microcirculation and has been associated with adverse cognitive outcomes. We sought to determine the relationship between central artery stiffness, brain arterial diameters, and white matter hyperintensity (WMH) volume in the general population of older adults. Approach and Results: We examined data from 1275 stroke-free participants of the ARIC (Atherosclerosis Risk in Communities) study (59% female, 30% Black, mean age 77 years) who underwent carotid-femoral pulse wave velocity (cf-PWV) testing and brain magnetic resonance imaging. A cf-PWV >12 m/s was considered elevated. Relationships between WMH and arterial diameters at the M1A1 segment of the middle and anterior cerebral arteries (whichever larger) were analyzed in the absence of artery-specific plaque, adjusting for intracranial volume and potential confounders. The average cf-PWV was 11.7 m/s and WMH volume was 11.2 cm 3 . Each 0.5 mm increment in M1A1 diameter was associated with 24% greater WMH volume in participants with elevated cf-PWV (exponentiated β=1.24 [95% CI, 1.12–1.36]), compared with a 10% greater WMH volume in participants without elevated cf-PWV (exponentiated β=1.10 [95% CI, 1.01–1.19]), P value for interaction=0.04. Conclusions: In this examination of older adults from the general population, larger, plaque-free arterial diameters in the middle cerebral circulation were associated with greater WMH volume, particularly among participants with elevated central artery stiffness. Strategies to reduce central artery stiffness may mitigate the association between cerebrovascular outward remodeling and microvascular damage.

Hepatopulmonary syndrome (HPS) markedly increases the mortality of patients. However, its pathogenesis remains incompletely understood. Rat HPS develops in common bile duct ligation (CBDL)-induced, but not thioacetamide (TAA)-induced cirrhosis. We investigated the mechanisms of HPS by comparing these two models. Pulmonary histology, blood gas exchange, and the related signals regulating macrophage accumulation were assessed in CBDL and TAA rats. Anti-polymorphonuclear leukocyte (antiPMN) and anti-granulocyte-macrophage colony stimulating factor (antiGM-CSF) antibodies, clodronate liposomes (CL), and monocyte chemoattractant protein 1 (MCP1) inhibitor (bindarit) were administrated in CBDL rats, GM-CSF, and MCP1 were administrated in bone marrow-derived macrophages (BMDMs). Pulmonary inflammatory cell recruitment, vascular dilatation, and hypoxemia were progressively developed by 1 week after CBDL, but only occurred at 4 week after TAA. Neutrophils were the primary inflammatory cells within 3 weeks after CBDL and at 4 week after TAA. M2 macrophages were the primary inflammatory cells, meantime, pulmonary fibrosis, GM-CSFR, and CCR2 were specifically increased from 4 week after CBDL. AntiPMN antibody treatment decreased neutrophil and macrophage accumulation, CL or the combination of antiGM-CSF antibody and bindarit treatment decreased macrophage recruitment, resulting in pulmonary fibrosis, vascular dilatation, and hypoxemia in CBDL rats alleviated. The combination treatment of GM-CSF and MCP1 promoted cell migration, M2 macrophage differentiation, and transforming growth factor-β1 (TGF-β1) production in BMDMs. Conclusively, our results highlight neutrophil recruitment mediates pulmonary vascular dilatation and hypoxemia in the early stage of rat HPS. Further, M2 macrophage accumulation induced by GM-CSF/GM-CSFR and MCP1/CCR2 leads to pulmonary fibrosis and promotes vascular dilatation and hypoxemia, as a result, HPS develops.

Background Concomitant corneal ectasia and posterior lamellar corneal opacification is rare, and the genetic relationship between these two conditions is unclear. We report the genetic and clinical characterization of this phenotype in three unrelated individuals. Materials and methods One previously reported affected individual and two unreported, unrelated, affected individuals were recruited for the study. Subjects and unaffected relatives underwent slit lamp examination, refraction, and multi-modal imaging. Saliva samples were obtained from two of the three affected individuals, from which DNA was extracted. Sanger sequencing was performed to identify mutations in genes associated with posterior amorphous corneal dystrophy (PACD), brittle cornea syndrome (BCS), and posterior polymorphous corneal dystrophy (PPCD), while copy number variation (CNV) analysis was used to identify CNV in the PACD locus. Results Affected individuals demonstrated bilateral corneal steepening, stromal thinning and lamellar posterior corneal opacification. Corneal topography and tomography revealed conical or globular corneal steepening and decreased thickness. Anterior segment optical coherence tomography demonstrated hyperreflectivity of the posterior stroma in each of the affected individuals. Genetic testing did not detect a heterozygous deletion involving the PACD locus on chromosome 12 or a pathogenic mutation in the genes associated with BCS or PPCD. Conclusions Corneal ectasia may be associated with posterior lamellar stromal opacification that appears consistent with PACD. However, genetic testing for PACD as well as BCS and PPCD in affected individuals fails to reveal pathogenic deletions or mutations, indicating that other genetic factors are involved.

Advances in medical care have led to more premature babies surviving the neonatal period. In these babies, germinal matrix haemorrhage (GMH), intraventricular haemorrhage (IVH) and posthaemorrhagic ventricular dilatation (PHVD) are the most important determinants of long-term cognitive and developmental outcomes. In this review, we discuss current neurosurgical management of IVH and PHVD, including the importance of early diagnosis of PHVD, thresholds for intervention, options for early management through the use of temporising measures and subsequent definitive CSF diversion. We also discuss treatment options for the evolving paradigm to manage intraventricular blood and its breakdown products. We review the evidence for techniques such as drainage, irrigation, fibrinolytic therapy (DRIFT) and neuroendoscopic lavage in the context of optimising cognitive, neurodevelopmental and quality of life outcomes in these premature infants.

Purpose of review Corneal collagen crosslinking (CXL) is a minimally invasive treatment that can stabilize corneal ectatic disorders including keratoconus, pellucid marginal degeneration, or postrefractive surgery ectasia. The benefits of CXL have been well documented. New research is focused on modifying current treatment protocols with the goals of maximizing corneal stability while also shortening overall procedure time. Recent findings Accelerated CXL protocols have the goal of delivering the same ultraviolet A intensity as conventional protocols, but over a shorter time period. Accelerated protocols have shown success to date, but there are concerns for long-term corneal stability. Pulsed protocols may increase the long-term efficacy of the accelerated designs. In addition, transepithelial crosslinking protocols have been designed with the goal of reducing postoperative pain and lower the risk of infectious complications of epithelial-off conventional protocols. Summary Newer CXL protocols attempt to make the procedure safer and more effective. Current research is promising, but long-term studies are essential to understand how the new protocols may affect corneal stability.

INTRODUCTION Several techniques have been described to taper the dilated small bowel to improve intestinal motility and decrease complications related to overdilated small bowel, including longitudinal intestinal lengthening and tapering, serial transverse enteroplasty, and spiral intestinal lengthening and tailoring. We propose an alternative technique designed to optimize bowel function and minimize the effects of recurrent small-bowel bacterial overgrowth in patients with short or ultra-short gut syndrome and dysfunctional anastomosis with maintenance of the actual absorptive surface. TECHNIQUE The dilated side-to-side anastomosis is identified, and the mesentery leaves from both the proximal and distal small-bowel loops are separated by using blunt dissection. The previous anastomosis is divided longitudinally with a GI stapler. Once the small-bowel transection is completed, 2 separate blind loops of intestine are created, each one with half the circumference of the dilated side-to-side anastomosis. The antimesenteric stapled line is then reinforced with an outer layer of running suture. The blind loops of the tapered small bowel are then trimmed and anastomosed in an end-to-end isoperistaltic fashion in 2 layers. RESULTS There were no postoperative complications. The length of the tapering ranged from 10 to 23 cm, corresponding to approximately 16% (range, 13%-20%) of the remaining small-bowel length. Three of 4 patients presented significant improvement of their symptoms and were able to have their parenteral support discontinued. CONCLUSIONS Modified antimesenteric tapering enteroplasty is an alternative technique to improve intestinal motility and treat patients with short-bowel syndrome and dysfunctional side-to-side anastomosis without the need for further small-bowel resection. This bowel-sparing technique represents a valuable option in the armamentarium of the surgeon who manages patients with intestinal failure.

Purpose To investigate the dilated choroidal veins (DCVs) at or around myopic macular neovascularizations (MNVs) and to determine whether there is a hemodynamic relationship between them. Methods Fifty-eight eyes of 57 patients with myopic MNVs were examined. Dilated choroidal veins were defined as choroidal veins whose diameter was 2X larger than adjacent veins. Indocyanine green angiography and swept-source optical coherence tomography images were reviewed to detect DCVs that crossed the subfoveal area. The filling sequence of the DCVs and MNVs was determined. Results Patients' mean age was 71.4 ± 10.6 years. The mean axial length was 29.3 ± 1.8 mm. Dilated choroidal veins below or around the MNV were found in 17 eyes (29.3%). Emissaries of the short posterior ciliary arteries were seen at or around MNVs in 8 of the 17 eyes. In these eyes, the short posterior ciliary artery was filled first or almost simultaneously with the filling of the MNV, followed by a laminar filling of the DCVs. In one eye, afferent arterioles from the short posterior ciliary arteries and efferent venules connected to DCVs were seen. Conclusion Dilated choroidal veins are present below or around MNVs in about 30% of eyes with myopic MNVs. Our findings suggest that an MNV might be a vascular unit consisting of short posterior ciliary arteries, afferent arterioles, efferent venules, and DCVs.

American Cocker Spaniels (ACSs) develop aural ceruminous gland hyperplasia and ectasia more often than dogs of other breeds. Data on the cause and development of these breed characteristic histopathological changes are lacking. We performed video-otoscopic examinations and dermatological work-up on 28 ACSs, obtained aural biopsies from each dog and assessed the statistical associations between the presence of ceruminous gland hyperplasia and ectasia and disease history, clinical or microbiological findings and underlying cause of otitis externa (OE). Histological lesions of ceruminous gland hyperplasia and ectasia were observed in aural biopsies from 6/13 clinically healthy ears and 13/15 ears with OE from 19/28 examined dogs. Nine of 28 dogs had histologically normal ceruminous glands (odds ratio [OR] 6.2, 95% confidence interval [CI] 1.1-36.6). Bacterial growth in microbiological culture of aural exudate (OR 14.1, 95% CI 2.1-95.3) was associated with ceruminous glandular changes, whereas previous history of OE, cutaneous findings or underlying allergies were not. Pedigree analysis and a genome-wide association study (GWAS) were performed on 18 affected and eight unaffected dogs based on histopathological diagnosis. While the GWAS indicated a tentative, but not statistically significant, association of ceruminous gland hyperplasia and ectasia with chromosome 31, a larger cohort is needed to confirm this preliminary result. Based on our results, ceruminous gland hyperplasia and ectasia may also precede clinical signs of OE in ACSs and a genetic aetiological component is likely Further studies with larger cohorts are warranted to verify our preliminary results. (C) 2021 The Authors. Published by Elsevier Ltd.

PURPOSE: To summarize the indications and outcomes of posterior chamber phakic intraocular lens (PIOL) implantation in corneal ectasias including keratoconus, pellucid marginal degeneration (PMD), post-refractive surgery, and post-keratoplasty ectasias. METHODS: A review of the literature was conducted using the relevant keywords from various databases up to August 15, 2020. All pertinent studies were reviewed, and the relevant articles were studied in detail for efficacy, stability, predictability, and safety outcomes. In addition, visual quality, corneal biomechanical outcomes, complications, the role of posterior chamber PIOL in combination treatment, and comparison of posterior chamber PIOL with other PIOLs for ectasias were also evaluated. RESULTS: A total of 30 relevant studies (13 prospective, 13 retrospective, 4 case reports) on the subject were studied and summarized. All studies showed a favorable refractive outcome. Quality of vision remained unaffected and no significant complications were reported in any of the studies. CONCLUSIONS: Posterior chamber PIOLs represent a viable option in the treatment of mild to moderate and stable corneal ectasia in patients with contact lens intolerance who have low irregular astigmatism, a clear central cornea, and good preoperative corrected distance visual acuity. [ J Refract Surg . 2021;37(5):351–359.]

International audience; Purpose: To evaluate the outcome of SPOT scleral lenses in the management of irregular astigmatism in patients with corneal ectasia and penetrating keratoplasty. Second, we analyzed patients' characteristics and tolerance, comfort, and geometries of fitted lenses.Method: Over a 5-year period, we included patients experiencing irregular astigmatism fitted with SPOT scleral contact lenses, from the University Hospital of Clermont-Ferrand, France. Data collected included corneal diseases, refractive error, best-corrected visual acuity (VA) with SPOT lenses, geometry of the lens, number of adjustment consultations, and the duration of follow-up. Comfort, quality of vision, less handling, and satisfaction were evaluated using visual analog scales after a 6-month follow-up period.Results: Sixty-five patients were included, analyzing 107 eyes. Eighty percent of patients still daily wore lenses after a follow-up of 22.3±13.8 months. Visual acuity improved by 0.47±0.51 logarithm of the minimum angle of resolution (average increase of 5 lines) (P75/100). Contact lenses were daily worn 10.0±4.1 hr/day. Most patients wore size M (17 mm) lenses (53.3% of patients), with an average sagittal height of 5.2±1.2 mm. Internal toricity was used in 30% of cases. Best geometry was found after 2.69±0.87 consultations.Conclusion: SPOT scleral contact lenses are an effective and well tolerated method to improve the VA of patients with irregular astigmatism.

Background Spontaneous spinal epidural hematoma (SSEH) is rare in children. Vascular malformation including arteriovenous fistulas and venous malformation is a rare cause of SSEH. Case description A 5-year-old girl presented with 2 episodes of SSEH at the upper thoracic spine and non-hemorrhagic episodes with spontaneous neurological recovery. Diagnostic study with MRI and spinal angiography demonstrated an unusual epidural arteriovenous fistula (AVF) with venous ectasia similar to venous malformation. She underwent embolization of the AVF with NBCA with mild transient neurological deterioration. Follow up angiography showed persistent occlusion of the embolized fistula and inconsistent visualization of another AVF to the patent venous ectasia. Conclusions This type of epidural AVF seems to be more common in children and tends to cause multiple neurologic episodes due to SSEH, venous expansion or thrombosis. Endovascular embolization with NBCA should be the first choice of treatment for this disease, unless emergent hematoma evacuation is necessary. Embolization should target at only the fistula site without significant penetration into the venous ectasia. Follow up is necessary for potential reappearance of AVF, even if AVF is occluded at the time of treatment. Time resolved MRI is useful to detect AVFs, thus for diagnosis and follow up of this disease.

Coronary ectasia (CE) is defined as dilation of the coronary artery, 1.5 times that of the surrounding vessel. Outcomes of percutaneous intervention (PCI) in patients with CE presenting as ST-elevated myocardial infarction (STEMI) remain a topic of debate.Studies comparing outcomes of PCI in CE versus no-ectasia (NE) STEMI patients were identified. Baseline angiographic characteristics include thrombolysis in myocardial infarction (TIMI) 0-1 flow, right coronary artery (RCA) involvement, and primary outcomes including thrombus aspiration, no-reflow, mortality, and TIMI-3 post-PCI. Odds ratio (OR) and 95% confidence interval (CI) were calculated.Six studies (n = 5746, CE-340 and NE-5406) qualified for the analysis. RCA involvement was more common in CE than NE, OR-1.39 (95%CI1.06-1.82, p-0.02). Pre-procedure TIMI-0-1 was of comparable results between the groups (p-1.13). Higher thrombus aspiration for CE (OR 2.18, 95%CI1.44-3.32;p-0.001). CE had higher incidence of no-reflow (OR 4.07, 95%CI2.42-6.84;p-0.001). TIMI-3 flow post-PCI was achieved less commonly in the CE group (OR-0.64, 95%CI-0.48-0.86;p-0.001). Mortality on follow-up was comparable (0.83, 95%CI0.39-1.78;p-0.63). Metaregression analysis did not show confounding effects from comorbidities.Coronary ectasia patients with STEMI had higher rates of PCI failure and no-reflow than NE; however, mortality during follow-up was comparable.

PURPOSE To quantify the effect of a single scleral lens design on visual acuity and ocular higher-order aberrations in eyes with post-LASIK ectasia, keratoconus and pellucid marginal degeneration (PMD) that could not achieve satisfactory vision with spectacles or soft contact lenses. METHODS Forty-six eyes of 28 participants fitted with diagnostic scleral lenses (KeraCare) were analysed, including 19, 15 and 12 eyes with post-LASIK ectasia, keratoconus and PMD, respectively. Corrected distance visual acuity (CDVA) and ocular aberrations were measured prior to lens wear and during lens wear after 60 min of settling. An i-Trace aberrometer was used to determine aberrations over a 4.5 mm diameter pupil. RESULTS Before lens wear, the median (95% confidence interval) values across all groups were: CDVA 0.30 (0.30, 0.40) logMAR, spherical equivalent refraction -2.75 (-5.25, -2.12) D, cylindrical refraction 3.75 (2.50, 5.00) D, higher-order-root-mean-square error (HO-RMS) 0.90 (0.64, 1.03) μm and vertical coma co-efficient C(3,-1) -0.32 (-0.42, -0.12) μm. RMS coma of 0.52 (0.40, 0.74) μm was higher for the keratoconus group than for the other groups (p

Objective In chronic aortic insufficiency (AI), the method and degree of annular downsizing required to achieve durable coaptation in aortic valve repair (AVr) remains poorly defined. This study evaluated the relationship between leaflet size and annular diameter to predict adequate annular sizing in remodeling AVr. Methods Under regulatory supervision, 74 patients with chronic tri-leaflet AI underwent AVr using ring annuloplasty and leaflet reconstruction. Fifty-four (73%) had ascending aortic ( n = 25) and/or root ( n = 29) aneurysms, and aortic grafts were sized 5 to 7 mm larger than the rings. Intraoperatively, leaflet free-edge length (FEL) was measured with special ball sizers positioned in the coronary sinus, and “normal” annular diameter was predicted from the validated formula: Required “normal” diameter = FEL/1.5. “Normal” annular diameters predicted from FEL were compared with pathologic diameters measured intraoperatively with Hegar dilators, and both were correlated with gender, age, and BSA. Results Average age was 62.1 ± 13.3 years (mean ± SD), 73% (54/74) were male, and 96% (71/74) had moderate-to-severe AI. All patients had annular dilatation, with a pathologic diameter 26.6 ± 2.3 mm before repair, and a predicted “normal” diameter of 21.7 ± 1.7 mm ( P < 0.001). Both predicted and pathologic annular diameters were larger in men ( P < 0.001), but no relationship existed with age. BSA correlated with both predicted and pathologic diameters, although variability was large. Conclusions Based on a simple validated method to predict “normal” annular diameter, all patients with chronic AI have some degree of annular dilatation. This finding implies that most AVr should include annuloplasty, with adequate and precise annular reduction based on leaflet size.

PURPOSE To point out the biomechanical changes of the topographically and tomographically normal partner eye (NPE) in patients with very asymmetrical corneal ectasia. SETTING Department of Ophthalmology, Saarland University Medical Center in Homburg/Saar, Germany. DESIGN Retrospective study. METHODS The topographical and tomographical results of the NPE were assessed using the Pentacam HR and the biomechanical corneal properties using the Ocular Response Analyzer (keratoconus match index [KMI], corneal hysteresis [CH], and corneal resistance factor [CRF]) and the Corvis ST (topographic biomechanical index [TBI] and Corvis biomechanical index) and compared those results with a normal control group (CG). RESULTS The clinical records of 26 patients recruited from the Homburg Keratoconus Center diagnosed with a very asymmetrical corneal ectasia were reviewed. The NPE (8.5 ± 1.5 mm Hg) showed a significantly more pathological CH (P < .001) compared with the CG. The CRF was also significantly more pathological (P = .04) for the NPE (8.3 ± 1.5 mm Hg) compared with the CG. The NPE (0.62 ± 0.32) showed a nonsignificant (P = .08) more pathological KMI compared with the CG. Nineteen (73.1%) of 26 NPE had a KMI less than 0.72 and were considered pathological. Compared with the CG, the TBI of the NPE (0.19 ± 0.25) did not differ significantly overall (P = .57). However, 5 (19.2%) of 26 eyes had a TBI more than 0.29 and were considered pathological. CONCLUSIONS Topographically and tomographically NPEs in very asymmetrical corneal ectasia frequently showed biomechanical changes. This should be considered before planning any type of refractive corneal surgery in such patients.

/Objectives: Endoscopic ultrasound elastography (EUS-EG) is useful for diagnosis of small solid pancreatic lesions (SPLs), particularly in excluding pancreatic cancer (PC), but its dependence on main pancreatic duct dilatation (MPDD) has not been examined. We aimed to investigate EUS-EG for diagnosis of small SPLs with and without MPDD.Patients with pathologically diagnosed SPLs of ≤20 mm were included and retrospectively analyzed. Using the blue:green ratio, an EUS-EG image was classified as blue-dominant, equivalent, or green-dominant. Using multiple EUS-EG images per patient, a lesion with a greater number of blue-dominant than green-dominant images was classified as stiff, and the others as soft. EUS-EG images in random order were judged by three raters. Considering stiff SPLs as PC, diagnostic performance of EUS-EG was examined for SPLs with and without MPDD.Of 126 cases analyzed, 65 (52%) were diagnosed as PC, and 63 (50%) had MPDD. A total of 1077 EUS-EG images were examined (kappa coefficient = 0.783). Lesions were classified as stiff in 91 cases and soft in 35 (kappa coefficient = 0.932). The ratio of stiff to soft lesions was significantly higher in PC than in non-PC (62:3 vs. 29:32, P 0.001). The sensitivity, specificity, and negative predictive value of a stiff lesion with vs. without MPDD for diagnosis of PC were 94%, 23%, and 50% vs. 100%, 60%, and 100%, respectively.Using the EUS-EG stiffness classification for small SPLs, PC can be excluded with high confidence and concordance for a soft lesion without MPDD.

Aortic root dilatation is a frequent disease affecting mostly young patients that often requires surgical repair. Surgical techniques in young patients include aortic valve-sparing procedures to avoid prosthetic valve implant.The aim of this paper is to describe the results obtained in three patients with aortic root dilatation using the Florida Sleeve technique.From November 2015 to January 2017, three patients with severe aortic regurgitation due to aortic anuloectasic were intervened applying the Florida Sleeve technique.Excellent postoperative results were obtained in the three cases including freedom of aortic regurgitation and any cause re-operation during three years of follow-up.The Florida Sleeve technique is a safe, reproducible technique with a learning curve and lower surgical times than traditional techniques. The medium-term clinical outcomes in terms of morbidity and mortality are good.La dilatación de la raíz aórtica es una patología frecuente que afecta a pacientes jóvenes y su tratamiento es quirúrgico. Las técnicas quirúrgicas de elección en pacientes jóvenes son las de preservación de la válvula aórtica para evitar el implante de una prótesis valvular.El presente trabajo tiene como objetivo describir la técnica de Florida Sleeve y los resultados de nuestra experiencia.Entre noviembre de 2015 y enero de 2017 fueron intervenidos en nuestro centro tres casos de pacientes con insuficiencia aórtica severa secundaria a anulectasia aórtica en los que se realizó la técnica de Florida Sleeve.En los tres casos se obtuvieron excelentes resultados postoperatorios, libres de insuficiencia aórtica residual y de reoperación por cualquier causa, resultados que se mantuvieron a los tres años de seguimiento.La técnica de Florida Sleeve es una técnica segura, reproducible con una curva de aprendizaje y tiempos quirúrgicos inferiores a las técnicas tradicionales. Los resultados clínicos a medio plazo en términos de morbimortalidad son buenos.

Objective To describe the technical aspects of robot assisted laparoscopic ureteral reimplantation (RALUR) for the management of primary obstructive megaureter (POM) and report initial outcomes, safety, and feasibility of the procedure. Methods Using an IRB- approved robotic surgery registry, we performed a retrospective chart review of patients undergoing RALUR for POM between April 2009 and May 2019. Results A total of 18 patients underwent RALUR using a modified Lich-Gregoir technique for management of POM and 7 (38.9%) of these underwent intracorporeal ureteral tapering at the time of surgery. At median follow up of 27.5 (IQR 11-50) months, no patient required reoperation for recurrent obstruction and all patients had improvement in hydronephrosis postoperatively. 30-day complications were low with 1 Grade I, 2 Grade II and 1 Grade III Clavien-Dindo complication. The most common issue postoperatively was febrile urinary tract infection, occurring in 6 patients (33.3%), at an average of 3.2 months after surgery. Increased operative time was the only significant difference between the tapered verses nontapered group. Conclusion We present the largest series of RALUR for POM to date. Based upon our initial experience with this technique we believe it is technically feasible and reproducible with good outcomes and low complication rates. Future studies are needed to track long-term outcomes and better understand indications for and the utility of ureteral tapering as well as how to minimize febrile UTIs postoperatively. Additional follow up is needed to determine the efficacy of RALUR as compared to open ureteral reimplantation for POM.

Background and aim Aortic aneurysms most commonly occur in the infra-renal and proximal thoracic regions. While generally asymptomatic, progressive aneurysmal dilation can become rapidly lethal when dissection or ruptures occurs, highlighting the need for more robust screening. Abdominal aortic aneurysm (AAA) is more prevalent compared to thoracic aortic aneurysm (TAA). The true incidence of TAA is underreported due to the absence of population screening and the silent nature of TAA. To achieve the optimum survival rate in aortic aneurysms, knowledge of natural course, genetic association, and surgical results are needed to be applied with adequate medical treatment and careful selection of patients for operation. The purpose of this paper is to provide a comprehensive review of the literature on natural history, immunology, and genetic differences between thoracic and AAAs. Method The literature was collected from OVID, SCOPUS, and PubMed. Results (1) AAA expands faster than TAA. AAA expands at approximately 0.3-0.45 cm annually, depending on various factors (advancing age, diameter of aorta, smoking etc.). TAA expands up to 0.3 cm annually in a non-bicuspid aortic valve patient. (2) An increase in Matrix metallopeptidase 1, 2, 9, 12, 14 led to degrading extracellular matrix of the aortic vessel wall. This significantly contributed to the pathogenesis in AAA, whereas overactive Transforming growth factor-beta played a major role in the pathogenesis of TAA. Conclusion In the future, genetic testing may be the gold standard for tackling the geneticheterogeneity of aneurysms, therefore, identifying at-risk individuals developing TAA andAAA earlier.

Hypomelanosis of Ito is a rare heterogeneous neurocutaneous disorder often associated with central nervous and musculoskeletal system involvement. Herein, we report the first case of hypomelanosis of Ito in the literature presenting with unilateral dilation of Virchow-Robin spaces (VRS). A girl aged 16 years old presented with a 1-year history of headache. Her physical and neurological examinations were normal, except for the presence of unilateral cutaneous macular hypopigmented whorls and streaks on lower side of the right trunk and lower limb, termed as Blaschko's lines. She had mild deficits in cognitive and adaptive functioning. Hearing, renal, dental, ophthalmologic, metabolic, and cardiac assessments were normal. Brain magnetic resonance imaging (MRI) showed markedly unilateral hemispheric enlarged VRS without contrast enhancement and diffusion restriction. To the best of our knowledge, our case is the first report describing the unilateral hemispheric enlarged VRS in a patient with hypomelanosis of Ito. Our report suggested that hypomelanosis of Ito may have unilateral dilation of VRS in brain MRI.

An infundibulum of the posterior communicating artery (PcomA) or infundibular dilatation (ID) is considered to be a benign anatomical variant. However, some evidence suggests that ID is a pre-aneurysmal state. This case series presents cases of ruptured IDs and aneurysms originating from the IDs. Between April 2002 and June 2020, 1337 patients were treated for aneurysmal subarachnoid hemorrhage (SAH). Among them, 7 patients with ruptured PcomA IDs were identified. Rupture IDs of the PcomA were categorized into 1) direct rupture of IDs, and 2) rupture of aneurysms originating from IDs. The clinical and radiographic factors were retrospectively reviewed. Two patients experienced direct rupture of IDs, while the other 5 patients presented with ruptured aneurysms originating from IDs. The 2 patients with direct rupture of IDs at presentation were relatively younger (< 50 years), while the other 5 patients with ruptured aneurysms originating from IDs were over 70 and had a history of aneurysmal SAH due to other intracranial aneurysms. Ruptured IDs were treated with microsurgery or endovascular treatment. There were no complications related to the procedure for securing ruptured IDs. Five (71.4%) patients showed favorable outcomes. One patient with initial Hunt and Hess grade 4 died due to initial brain damage with cerebral vasospasm and medical complications. ID of the PcomA rarely causes SAH with or without aneurysm formation. Thus, patients with IDs of the PcomA should be carefully followed up for a long period, especially those with a history of aneurysmal SAH.

Importance Active pediatric COVID-19 pneumonia and MIS-C are two disease processes requiring rapid diagnosis and different treatment protocols. Objective To distinguish active pediatric COVID-19 pneumonia and MIS-C using presenting signs and symptoms, patient characteristics, and laboratory values. Design Patients diagnosed and hospitalized with active COVID-19 pneumonia or MIS-C at Children’s of Alabama Hospital in Birmingham, AL from April 1 through September 1, 2020 were identified retrospectively. Active COVID-19 and MIS-C cases were defined using diagnostic codes and verified for accuracy using current US Centers for Disease Control case definitions. All clinical notes were reviewed for documentation of COVID-19 pneumonia or MIS-C, and clinical notes and electronic medical records were reviewed for patient demographics, presenting signs and symptoms, prior exposure to or testing for the SARS-CoV-2 virus, laboratory data, imaging, treatment modalities and response to treatment. Findings 111 patients were identified, with 74 classified as mild COVID-19, 8 patients as moderate COVID-19, 8 patients as severe COVID-19, 10 as mild MIS-C and 11 as severe MIS-C. All groups had a male predominance, with Black and Hispanic patients overrepresented as compared to the demographics of Alabama. Most MIS-C patients were healthy at baseline, with most COVID-19 patients having at least one underlying illness. Fever, rash, conjunctivitis, and gastrointestinal symptoms were predominant in the MIS-C population whereas COVID-19 patients presented with predominantly respiratory symptoms. The two groups were similar in duration of symptomatic prodrome and exposure history to the SARS-CoV-2 virus, but MIS-C patients had a longer duration between presentation and exposure history. COVID-19 patients were more likely to have a positive SAR-CoV-2 PCR and to require respiratory support on admission. MIS-C patients had lower sodium levels, higher levels of C-reactive protein, erythrocyte sedimentation rate, d-dimer and procalcitonin. COVID-19 patients had higher lactate dehydrogenase levels on admission. MIS-C patients had coronary artery changes on echocardiography more often than COVID-19 patients. Conclusions and relevance This study is one of the first to directly compare COVID-19 and MIS-C in the pediatric population. The significant differences found between symptoms at presentation, demographics, and laboratory findings will aide health-care providers in distinguishing the two disease entities.

Maturation failure remains a major clinical problem of distal arteriovenous fistula (AVF). Early failure (EF) is associated with the small size of the veins. For about 10 years we have used in more than 1000 fistulas, the Vessels Pre-Dilatation (VPD) to increase the recruitment of small veins for creating distal AVFs. The purpose of this study is to highlight if the VPD can reduce the incidence of EF or failure to mature (FTM) in AVFs created with small veins. Data of all the consecutive patients directly admitted to our Department for their first distal AVF from January to December 2019 were collected. The patients were divided in two groups, one with a vein diameter after the tourniquet ⩽2.0 mm (G1) and one >2 mm (G2). Both in G1 then in G2 the vessels had undergone VPD. Immediate failure (IF), EF, FTM, delayed or arrested maturation rate (DAM), unassisted AVFs and matured AFVs were evaluated. The patients recruited totalled 104, 37 in G1, and 67 in G2. The two groups were homogeneous in age, incidence of diabetes, obesity, heart disease, peripheral vasculopathy, and race. Female were more numerous in G1 (51% vs 12%, p